title: Nutritional practices at a glance: spinal muscular atrophy type I nutrition survey findings.
abstract: Proactive nutritional management for children with spinal muscular atrophy type I can provide insight into improved spinal muscular atrophy care. This observational study consisted of a nutritional and medical history survey of children with spinal muscular atrophy type I collected in 2009-2011. Forty-four caregiver survey responses were evaluated using descriptive statistics. Average age of spinal muscular atrophy type I subjects was 5 years (5 mo-16 y). The subject cohort was composed of 22 males, 21 females, and 1 unreported. Nutrition support via feeding tube was utilized by 43 of 44 subjects. A majority of respondents reported using elemental or semi-elemental formula for subjects' essential caloric intake (34 of 44). Formula intolerance issues were reported by many caregivers (27 of 44). Half of caregivers implemented dietary changes on their own or with guidance from other families; 15 caregivers consulted a registered dietitian. Survey responses and comments indicate need for evidence-based nutritional guidelines for spinal muscular atrophy.
authors.affiliation: Pediatric Motor Disorders Research Program, University of Utah, Department of Neurology, Salt Lake City, UT, USA rhurst@genetics.utah.edu.
authors.forename: Rebecca Hurst
authors.initials: RH
authors.lastname: Davis
authors.affiliation: Division of Nutrition Therapy, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Barbara J
authors.initials: BJ
authors.lastname: Godshall
authors.affiliation: University of Wisconsin Pediatric Pulmonary Center, University of Wisconsin School of Medicine and Public Health, American Family Children's Hospital, Madison, WI, USA.
authors.forename: Erin
authors.initials: E
authors.lastname: Seffrood
authors.affiliation: University of Wisconsin Pediatric Pulmonary Center, University of Wisconsin School of Medicine and Public Health, American Family Children's Hospital, Madison, WI, USA.
authors.forename: Mary
authors.initials: M
authors.lastname: Marcus
authors.affiliation: Biomedical Research Informatics Service Core, University of Utah, Salt Lake City, UT, USA.
authors.forename: Bernard A
authors.initials: BA
authors.lastname: LaSalle
authors.affiliation: Pediatric Neuromuscular Program, Comprehensive Neuromuscular Center, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
authors.forename: Brenda
authors.initials: B
authors.lastname: Wong
authors.affiliation: University of Wisconsin Pediatric Pulmonary Center, University of Wisconsin School of Medicine and Public Health, American Family Children's Hospital, Madison, WI, USA.
authors.forename: Mary K
authors.initials: MK
authors.lastname: Schroth
authors.affiliation: Pediatric Motor Disorders Research Program, University of Utah, Department of Neurology, Salt Lake City, UT, USA.
authors.forename: Kathryn J
authors.initials: KJ
authors.lastname: Swoboda
mesh_terms: D000293:Adolescent; D017028:Caregivers; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D003729:Dental Care / Q000379:methods; D004750:Enteral Nutrition; D005260:Female; D005526:Food, Formulated; D005765:Gastrointestinal Agents / Q000627:therapeutic use; D006801:Humans; D007223:Infant; D008297:Male; D009749:Nutrition Surveys; D012138:Respiratory Therapy; D014897:Spinal Muscular Atrophies of Childhood / Q000178:diet therapy* / Q000453:epidemiology* / Q000628:therapy
chemical_list: D005765:Gastrointestinal Agents
keywords: SMA nutrition; elemental diet; spinal muscular atrophy type I